Trends in Pediatric Intestinal Failure: A Multicenter, Multinational Study

Daniela Gattini, Amin J. Roberts, Paul W. Wales, Sue V. Beath, Helen M. Evans, Jonathan Hind, David Mercer, Theodoric Wong, Jason Yap, Christina Belza, Koen Huysentruyt, Yaron Avitzur

Research output: Contribution to journalArticlepeer-review

4 Scopus citations

Abstract

Objectives: To assess the natural history and outcomes of children with intestinal failure in a large, multicenter, geographically diverse contemporary cohort (2010-2015) from 6 pediatric intestinal failure programs. Study design: Retrospective analysis of a multicenter intestinal failure cohort (n = 443). Competing-risk analysis was used to obtain cumulative incidence rates for the primary outcome (enteral autonomy, transplantation, or death). The χ2 test and Cox proportional hazard regression were used for bivariate and multivariable analyses. Results: The study cohort comprised 443 patients (61.2% male). Primary etiologies included short bowel syndrome (SBS), 84.9%; dysmotility disorder, 7.2%; and mucosal enteropathy, 7.9%. Cumulative incidences for enteral autonomy, transplantation, and death at 6 years of follow-up were 53.0%, 16.7%, and 10.5%, respectively. Enteral autonomy was associated with SBS, ≥50% of small bowel length, presence of an ileocecal valve (ICV), absence of portal hypertension, and follow-up in a non–high-volume transplantation center. The composite outcome of transplantation/death was associated with persistent advanced cholestasis and hypoalbuminemia; age <1 year at diagnosis, ICV, and intact colon were protective. Conclusions: The rates of death and transplantation in children with intestinal failure have decreased; however, the number of children achieving enteral autonomy has not changed significantly, and a larger proportion of patients remain parenteral nutrition dependent. New strategies to achieve enteral autonomy are needed to improve patient outcomes.

Original languageEnglish (US)
Pages (from-to)16-23.e4
JournalJournal of Pediatrics
Volume237
DOIs
StatePublished - Oct 2021

Keywords

  • enteral autonomy
  • intestinal failure
  • intestinal transplantation

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

Fingerprint

Dive into the research topics of 'Trends in Pediatric Intestinal Failure: A Multicenter, Multinational Study'. Together they form a unique fingerprint.

Cite this